Importance of Adequate Surgical Local Control in Fusion-Negative Para-Testicular Rhabdomyosarcoma: Data From the Cooperative Weichteilsarkom Studiengruppe Trials (CWS-96 and CWS-2002P) and the European Soft Tissue Sarcoma Registry (SoTiSaR).

BACKGROUND: This study aimed to assess the impact that the quality of primary and subsequent surgeries has on the survival of patients with para-testicular rhabdomyosarcoma (PTRMS). METHODS: Patients with localized (IRS I-III) and metastatic (IRS IV) PTRMS were enrolled in the two Cooperative Weichteilsarkom Studiengruppe (CWS) trials (CWS-96, CWS-2002P) and the Soft Tissue Sarcoma Registry (SoTiSaR). RESULTS: Among 196 patients (median age, 8.4 years), 106 (54.1%) had primary complete resection. Image-defined lymph node (LN) disease was detected in 21 (11.5%) patients in the localized cohort and 12 (92.3%) patients in the metastatic cohort. The 5-year event-free survival (EFS) and overall survival (OS) were respectively 87.3% and 94.0% for the patients with localized PTRMS and 46.2% and 42.2% for the patients with metastatic PTRMS. Protocol violations during the primary surgery (PV-PS) were observed in 70 (42%) of the IRS I-III patients. This resulted in higher rates of R1/R2 resections (n = 53 [76%] vs n = 20 [21%]; p < 0.001) with a need for pretreatment re-excision (PRE) (n = 50 [83%] vs n = 10 [17%]; p < 0.001) compared with the patients undergoing correct primary surgery. Protocol violations during PRE occurred for 13 (20%) patients. Although PV-PS did not influence the 5-year EFS or OS in the localized PTRMS cohort, the unadjusted log-rank test showed that R status after PRE is a prognostic factor for 5-year OS (R1 vs R0 [81.8% vs 97.6%]; p = 0.02). CONCLUSIONS: The quality of surgical local control in PTRMS is unsatisfactory. Emphasis should be placed on evaluating the resection status after PRE in further clinical trials.

Robot-assisted retroperitoneal lymph node dissection: technique and initial case series of 18 patients.

OBJECTIVE: To evaluate outcomes of the first 18 patients treated with robot-assisted retroperitoneal lymph node dissection (RA-RPLND) for non-seminomatous germ cell tumours (NSGCT) and paratesticular rhabdomyosarcoma (RMS) at our institution. PATIENTS AND METHODS: Between March 2008 and May 2013, 17 patients underwent RA-RPLND for NSGCT and one for paratesticular RMS. Data were collected retrospectively on patient demographics, preoperative tumour characteristics, and perioperative outcomes including open conversion rate, lymph node (LN) yield, rate of positive LNs, operative time, estimated blood loss (EBL), and length of stay (LOS). Perioperative outcomes were compared between patients receiving primary RA-RPLND vs post-chemotherapy RA-RPLND. Medium-term outcomes of tumour recurrence rate and maintenance of antegrade ejaculation were recorded. RESULTS: RA-RPLND was completed robotically in 15 of 18 (83%) patients. LNs were positive in eight of 18 patients (44%). The mean LN yield was 22 LNs. For cases completed robotically, the mean operative time was 329 min, EBL was 103 mL, and LOS was 2.4 days. At a mean (range) follow-up of 22 (1-58) months, there were no retroperitoneal recurrences and two of 17 (12%) patients with NSGCT had pulmonary recurrences. Antegrade ejaculation was maintained in 91% of patients with a nerve-sparing approach. Patients receiving primary RA-RPLND had shorter operative times compared with those post-chemotherapy (311 vs 369 min, P = 0.03). There was no significant difference in LN yield (22 vs 18 LNs, P = 0.34), EBL (100 vs 313 mL, P = 0.13), or LOS (2.75 vs 2.2 days, P = 0.36). CONCLUSION: This initial selected case series of RA-RPLND shows that the procedure is safe, reproducible, and feasible for stage I-IIB NSGCT and RMS in the hands of experienced robotic surgeons. Larger studies are needed to confirm the diagnostic and therapeutic utility of this technique.

Lymph node management in patients with paratesticular rhabdomyosarcoma: a population-based analysis.

BACKGROUND: Paratesticular rhabdomyosarcoma (PTRMS) is the most common primary solid tumor arising from the mesenchymal tissue of the testis. Traditionally, retroperitoneal lymph node dissection is not recommended for children aged <10 years because of the morbidity of the procedure and low risk of retroperitoneal lymph node involvement. In the current study, the authors analyzed the patient and tumor characteristics of PTRMS as well as survival outcomes associated with lymph node dissection status. METHODS: A total of 255 cases of PTRMS were identified from the patient data reported by the Surveillance, Epidemiology, and End Results (SEER) program of the National Cancer Institute from 1973 through 2009. RESULTS: Among 173 patients aged ≥ 10 years, lymph node dissection was found to improve the 5-year overall survival (OS) rate from 64% to 86% (P < 0.01). Conversely, patients aged <10 years fared extremely well regardless of lymph node dissection status; the 5-year OS rate was 100% and 97%, respectively, for patients who did versus those who did not undergo lymph node dissection (P = .37). The yield of positive lymph nodes was approximately ≥ 20% when < 11 lymph nodes were removed. The incidence of lymph node involvement was also higher in older patients compared with younger patients (40% vs 8%). Radiotherapy improved the OS rate in patients with lymph node involvement (5-year OS rate: 90% with vs 36% without radiation; P < .0001). CONCLUSIONS: Lymph node dissection is recommended in patients aged ≥10 years. Radiotherapy is beneficial in patients with lymph node-positive disease.

Paratesticular rhabdomyosarcoma: a rare case report from Syria.

Paratesticular rhabdomyosarcoma (RMS) is a rare aggressive tumor manifesting in children and young adults. This tumor derives from mesenchymal elements of the Tunica vaginalis, epididymis, and spermatic cord. It is a very metastatic lesion that can spread by lymphatics to the iliac, para-aortic nodes, lung, and bone. Case presentation: In this paper, the authors report a case of a 6-year-old child who presented to the clinic with a painless mass in the right side of the scrotum. The mass was misdiagnosed and had evolved rapidly over 2 weeks. The mass measured 16×32 mm on ultrasound, and therefore, an orchiectomy was performed. The histological examination of the excised tissue confirmed the diagnosis of paratesticular RMS. Discussion: Paratesticular RMS mainly presented as a painless mass in the scrotum. It was a very metastatic lesion that required an immediate management. However, a lot of cases of paratesticular RMS misdiagnosed at first time, which worsens the overall prognosis. Conclusion: Eventually, paratesticular RMS should be always taken into consideration when a scrotal mass is suspected. Due to its extremely serious metastatic potential, this condition requires early diagnosis and management. The treatment is currently well codified combining surgery, chemotherapy, and radiotherapy.

Indocyanine green fluorescence-guided lymphadenectomy with single site retroperitoneoscopy in children.

BACKGROUND: Retroperitoneoscopic lymphadenectomy is an established surgical approach in adult urology, but rarely described in pediatric population. METHODS: We develop retroperitoneoscopic surgical oncology in children, combining new technology innovations in pediatric surgery such as single site port retroperitoneoscopic in supine position and indocyanine green (ICG). RESULTS: The video describes a step-by-step approach from the ICG injection technique to the lymph-node retroperitoneoscopic harvesting. The video highlights anatomical landmarks and ICG intraoperative lymph nodes findings. Four consecutive surgical procedures were performed in children with paratesticular rhabdomyosarcoma who required staging template retroperitoneal lymph node dissection (RPLND) for staging. All patients were discharged the same day without 30-days postoperative complications. CONCLUSION: Retroperitoneoscopic approach with single port and indocyanine guided lymphatic mapping for template retroperitoneal lymph node dissection (RPLND) is a feasible minimally invasive procedure in children. Combining different technology innovations allows an effective lymph node harvesting with the possibility to offer an enhanced recovery after surgery in pediatric oncology population.

Pediatric Rhabdomyosarcomas of the Genitourinary Tract.

Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in the pediatric and adolescent population, with 350 new cases diagnosed each year. While they can develop anywhere in the body, the genitourinary tract is the second most common primary location for an RMS to develop. Overall survival has improved through the increased use of protocols and multidisciplinary approaches. However, the guidelines for management continue to change as systemic and radiation therapeutics advance. Given the relative rarity of this disease compared to other non-solid childhood malignancies, healthcare providers not directly managing RMS may not be familiar with their presentation and updated management. This review aims to provide foundational knowledge of the management of RMSs with an emphasis on specific management paradigms for those arising from the genitourinary tract. The genitourinary tract is the second most common location for an RMS to develop but varies greatly in symptomology and survival depending on the organ of origin. As the clinical understanding of these tumors advances, treatment paradigms have evolved. Herein, we describe the breadth of presentations for genitourinary RMSs with diagnostic and treatment management considerations, incorporating the most recently available guidelines and societal consensus recommendations.

Surgical resection of a massive residual retroperitoneal mass after chemotherapy for a paratesticular rhabdomyosarcoma: a case report.

INTRODUCTION: Paratesticular rhabdomyosarcoma is a rare and aggressive mesenchymal tumor, accounting for only 7% of all rhabdomyosarcomas. It is mainly encountered in children and adolescents. The standard treatment consists of radical orchidectomy with negative surgical margins. However, chemotherapy is recommended to control retroperitoneal micrometastasis. The place of surgery for progressive retroperitoneal lymph node metastases remains controversial. We present a case of paratesticular rhabdomyosarcoma with progressive retroperitoneal lymph node metastases treated with surgery. CASE REPORT: We report a case of a 17-year-old North African male with no particular medical history who presented with a left scrotal mass that had been evolving for several months. Beta-human chorionic gonadotropin, alpha-fetoprotein, and lactate dehydrogenase were normal. Scrotal ultrasonography revealed the presence of a 6 cm heterogeneous hypoechogenic tissular mass with cystic areas adherent to the left scrotal wall, which was thickened in some places and vascularized by color Doppler. It exerted a mass effect on the homolateral testicle, which was of average volume. The thoracic-abdominal-pelvic computed tomography scan showed the presence of suspicious paraaortic lymph nodes. The most voluminous one measured 16 × 23 mm2. A left orchidectomy was performed. The final pathology report revealed an 8 cm paratesticular rhabdomyosarcoma of the embryonic type that displaced the testicle without invading it. Without going beyond it, it infiltrated the epididymis, the rete testis, and the albuginea. The surgical margin at the level of the spermatic cord was free. The patient had adjuvant chemotherapy (ifosfamide, vincristine, and dactinomycin). The patient had a challenging paraaortic lymph node dissection since the mass enlaced the left ureter and renal vessels. On histological examination, the paraaortic lymph nodes were metastatic. CONCLUSION: Rhabdomyosarcoma is an aggressive malignancy with high metastatic potential. Therefore, only an accurate diagnosis and early treatment can ensure better survival. Surgery in expert hands seems to be a good option for progressive retroperitoneal nodes. However, further studies are needed to determine the place of surgery in this setting.

Case Report: Paratesticular Rhabdomyosarcoma.

Paratesticular rhabdomyosarcoma (RMS) accounts for only 7% of all the RMS cases. Due to the limited available data, there is no consensus on the diagnosis and management of the paratesticular tumors. Here, we interrogated two paratesticular RMS cases in 25 and 27-year-old men presenting with painless and rapidly growing mass in the scrotum. Whereas the data showed no upregulation of tumor markers such as ß-human chorionic gonadotropin (ß-HCG), alpha-fetoprotein (AFP), and lactate dehydrogenase (LDH), scrotal ultrasonography and magnetic resonance imaging indicated the existence of paratesticular and inguinal lesions respectively. There was local recurrence in one patient who underwent radical orchiectomy for the sarcoma one year ago. In addition, the CT scans showed no occurrence of distant metastasis. The two patients underwent radical inguinal orchiectomy or resection of the recurrent tumors with nerve-sparing retroperitoneal lymph node dissection. Histologic examination revealed embryonal RMS (eRMS) without lymph node metastasis. We highlight the importance of multi-disciplinary participation for paratesticular RMS detection and preoperative ultrasound-guided needle biopsy (UNB) for rapid confirmatory diagnosis. Complete surgical resection coupled with chemotherapy and radiotherapy is the main treatment option for the paratesticular RMS. In addition, sperm cryopreservation treatment and endocrine follow-up could increase the overall survival and quality of life of the patients.

Alternative approaches to retroperitoneal lymph node dissection for paratesticular rhabdomyosarcoma.

PURPOSE: The aim of this study was to evaluate outcomes based on surgical approach for retroperitoneal lymph node dissection (RPLND) in patients with paratesticular rhabdomyosarcoma (PT-RMS). METHODS: Patients undergoing RPLND for PT-RMS over 10 years at a single institution were retrospectively reviewed. Length of stay (LOS), complications, oral morphine equivalents per kilogram (OME/Kg), lymph node yield, and time to chemotherapy were assessed. The surgical approaches compared were: open transabdominal, open extraperitoneal, laparoscopic, and retroperitoneoscopic. For cases with lymphatic mapping, indocyanine green (ICG) was injected into the spermatic cord. RESULTS: Twenty patients were included: five open transabdominal, six open extraperitoneal, three laparoscopic, and six retroperitoneoscopic operations. LOS was shorter in the retroperitoneoscopic group than laparoscopic (p = 0.029) and both open groups (p < 0.001). Mean OME/kg used was lowest in the retroperitoneoscopic (0.13 ±â€¯0.15) group compared to laparoscopic (0.68 ±â€¯0.53, p = 0.043), open transabdominal (14.90 ±â€¯8.87, p = 0.003), and extraperitoneal (10.11 ±â€¯2.44, p < 0.001). Time to chemotherapy was shorter for retroperitoneoscopic patients (0.13 days ±â€¯0.15) compared to open transabdominal (15.6 days±6.5, p = 0.005). There was no difference in lymph node yield between groups. Spermatic cord ICG demonstrated iliac lymph node avidity on near-infrared spectroscopy. CONCLUSIONS: Minimally invasive RPLND appears to offer a faster recovery without compromising lymph node yield for patients with PT-RMS. LEVEL OF EVIDENCE: III.

Paratesticular rhabdomyosarcoma: Importance of initial therapy.

PURPOSE: To evaluate factors associated with progression-free and disease-specific survival in patients with paratesticular rhabdomyosarcoma, we performed a cohort study. Also, since many patients present to our institution after initial therapy, we analyzed the effects of salvage therapy for scrotal violation. PATIENTS AND METHODS: We retrospectively reviewed the records of all consecutive patients with histologically confirmed paratesticular rhabdomyosarcoma treated at our institution between 1978 and 2015. Fifty-one patients were initially identified, but two with incomplete data were excluded from analysis. Variables evaluated for correlation with survival were TNM staging, Children's Oncology Group Soft Tissue Sarcoma pretreatment staging, margins at initial resection, presence of scrotal violation, hemiscrotectomy and/or scrotal radiation. The log-rank test was used to compare survival distributions. RESULTS: For the analytic cohort of 49 patients, the median age and follow-up were 15.7years (95% CI: 14.2-17.5, range: 0.8-25.1years) and 6.9years (95% CI: 4.4-9.0, range 0.2-37.5years), respectively. The 5-year overall disease-specific survival was 78.7% (95% CI: 67.7%-91.4%) and the progression-free survival was 66.9% (95% CI: 54.8%-81.6%). Median time to recurrence was 0.9years (95% CI: 0.7-0.9, range 0.1-6.2years). Scrotal violation occurred in 41% (n=20) and tripled the risk of recurrence for patients not appropriately treated with either hemiscrotectomy or scrotal radiation therapy (RR=3.0, 95% CI: 1.16-7.73). CONCLUSIONS: The strongest predictors of disease-specific survival were nodal status and distant metastasis at diagnosis. Scrotal violation remains a problem in paratesticular rhabdomyosarcoma and is a predictor of disease progression unless adequately treated. The risk of progression could be reduced with appropriate initial resection. LEVEL OF EVIDENCE: Level IV; retrospective study with no comparison group.

Quality assessment of lymph node sampling in rhabdomyosarcoma: A surveillance, epidemiology, and end results (SEER) program study.

BACKGROUND: Lymph node sampling is integral in the management of extremity and paratesticular rhabdomyosarcoma (RMS). The aim of this study was to determine overall surgical compliance with treatment protocols and impact of nodal sampling outcomes in these tumors. METHODS: A query of the surveillance, epidemiology, and end results program (SEER) database was performed from 2003 to 2008 for patients <19years of age with RMS. Data obtained included demographics, five-year survival and rate of nodal sampling. Analysis was performed utilizing chi-squared, Kaplan-Meier and hazard ratio modeling. RESULTS: Of 537 patients with extremity RMS, nodal sampling was performed in 25.7% (n=138). This lack of nodal sampling had a negative outcome on survival (p=0.004). Sixty five patients with paratesticular RMS aged greater than 10 were identified and also displayed low rates of lymph node sampling (47.7%, n=31). For paratesticular patients, a similar increase in survival was seen in patients who underwent nodal evaluation (p=0.024). CONCLUSION: Lymph node sampling is the standard of care in RMS. However, surgical compliance with treatment protocols is poor. Nodal evaluation correlated significantly with overall survival. These findings suggest a need for improved education among surgeons and oncologists regarding the need lymph node assessment in pediatric oncology patients. Evidence rating/classification: Prognosis study, Level III.

A Case of Long-term Survival of Advanced Paratesticular Rhabdomyosarcoma Treated With a Multimodal Therapy Including a Combination of Cyclophosphamide, Vincristine, Doxorubicin and Dacarbazine.

There is no established treatment for advanced rhabdomyosarcoma (RMS) with metastases at the time of diagnosis. A 17-year-old male was referred to our hospital because of a right scrotal mass. Computed tomography showed multiple lung metastases with pleural effusion and retroperitoneal lymph node metastasis, and bone scintigraphy revealed multiple bone metastases. Right high orchiectomy was performed and the tumor was diagnosed as paratesticular embryonal RMS. He was treated with a multimodal therapy including 17 cycles of combination chemotherapy consisting of cyclophosphamide, vincristine, doxorubicin and dacarbazine (CYVADIC) and achieved a long-term survival of 4 years.